DYSPLASIA EPIPHYSEALIS HEMIMELICA TREATED WITH OSTEOCHONDRAL ALLOGRAFT: A CASE REPORT. Chris A. Anthony MD, Brian R. Wolf MD, MS
ABSTRACT Background: Dysplasia epiphysealis hemimelica (DEH), or Trevor’s disease, is a developmental disorder of the pediatric skeleton characterized by asymmetric osteochondral overgrowth. Methods: We present the case of a five year old boy with a two year histor y of right knee pain and evidence of DEH on imaging who underwent initial arthroscopic resection of his lesion with subsequent recurrence. The patient then underwent osteochondral allograft revision surger y and was asymptomatic at two year follow-up with a congruent joint surface. Results: To our knowledge, this is the first reported case of a DEH lesion treated with osteochondral allograft and also the youngest reported case of osteochondral allograft placement in the literature. Conclusions: Osteochondral allograft may be a viable option in DEH and other deformities of the pediatric knee. Level of Evidence: Level V INTRODUCTION Dysplasia epiphysealis hemimelica (DEH), or Trevor’s disease, is a developmental disorder of the skeleton most often characterized by asymmetric osteochondral overgrowth1-3. DEH was initially reported in 1926 and was further described by Trevor in 19504,5. The disease is rare with a reported incidence of one per million and a male predominance with a male: female ratio ranging from 2:1 to 3:12,6. The distal femur, distal tibia and fibula, The Department of Orthopaedic Surgery and Rehabilitation, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, 01008 JPP, Iowa City, IA 52242, USA Email: [email protected] Phone: 563-343-1951 Each author certifies that his or her institution approved or waived approval for the human protocol for this investigation and that all investigations were conducted in conformity with ethical principles of research. Each author certifies that he or she, or a member of his or her immediate family, has no funding or commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the current article.
42 The Iowa Orthopaedic Journal
talus and calcaneus are most often affected with lesions involving the carpal bones, bones of the midfoot, scapula, acetabulum and elbow also reported2,7. DEH can affect either the medial or lateral epiphysis, though the medial epiphysis is most commonly involved1-3. Clinical findings typically include deformity, pain, limb-length discrepancy, muscle wasting, swelling, and limitations in joint range of motion2,3. Histologically the lesion is similar to an osteochondroma, however, DEH lacks the EXT1 and EXT2 gene mutations seen in osteochondroma8,9. DEH is also classically an epiphyseal lesion whereas osteochondroma is more commonly metaphyseal in location3. On plain radiographs the typical appearance of DEH is asymmetric epiphyseal cartilaginous overgrowth, containing more than one ossification center, with varying patterns of epiphyseal chondral calcification3. Computed tomography can be used for the detection of small foci of early calcification within the cartilaginous mass of a DEH lesion and can identify cortical and medullary continuity between the lesion and the neighboring bone3. Additionally, MRI can be useful in characterizing the size and dimensions of the lesion before it begins to ossifiy3. Management options of DEH include observation, surgical excision, and corrective osteotomy3, 10, 11. Surgical removal is indicated if the lesion is causing deformity, pain, compromising function, or resulting in an intraarticular loose body5, 12, 13. Osteochondral allograft (OCA) is an accepted method of treatment for restoring large chondral and osteochondral defects on the femoral condyles. Previous work has found survival rates of OCA in cases of idiopathic, focal chondral or osteochondral lesions of the femoral condyles ranged from 85%-100% at 5 years, 71%-89% at 10 years, 74%-76% at up to 15 years, and 66% at up to 20 years14-19. In cases of osteochondritis dissecans (OCD) and steroid associated osteonecrosis, OCA survival rates of 79% to 94% have been seen at less than five years follow-up19-23. There is limited data regarding OCA use in the pediatric knee. Published results have shown that 82% of patients returned to full sporting activities by twelve months24 and a graft survivorship of 90% at ten years25. We present the case of a five year old boy who underwent arthroscopic resection for a symptomatic left medial femoral condyle DEH lesion and subsequent
Dysplasia Epiphysealis Hemimelica Treated With Osteochondral Allograft
1a
1b
Figures 1a, 1b. Initial preoperative radiographs of the left knee which demonstrate an irregular radiodensity of the condylar surface along the posterior aspect of the medial femoral condyle.
2a
2b
Figures 2a, 2b. Radiographs of the articular surface of the left knee immediately after initial arthroscopic debridement of the DEH lesion. Of note, there is approximately 10% of the anterior portion of the lesion that was not removed as it was in the weightbearing zone of the medial femoral condyle.
repeat resection and placement of OCA due to lesion recurrence. The patient’s course with two year follow-up including history, exam, radiology findings, and treatment are discussed. CASE REPORT A five year old boy was referred to orthopaedics clinic by his local pediatrician with a two year history of worsening left knee pain and limp. Radiographs of the
knee were consistent with DEH of the left medial femoral condyle (Figures 1a, 1b). During initial arthroscopic resection, there was noted to be a prominence of the articular surface on the posterior aspect of the medial femoral condyle. The cartilage overlying the lesion was taken down with a shaver and the bony prominence within the epiphysis was identified and resected. Approximately 80-90% of the lesion was removed while the anterior-most portion of the lesion was left undisturbed Volume 35 43
C. A. Anthony, B. R. Wolf
3a
3b
Figures 3a, 3b. Radiographs demonstrating the recurrence of an irregular, crown shaped lesion along the posterior aspect of the medial femoral condyle at approximately 4 months after the index procedure.
A
4a
4b
Figures 4a, 4b. Immediate postoperative radiographs after placement of the adult osteochondral allograft.
as it was distinctly in the weightbearing zone of the knee (Figures 2a, 2b). At the patient’s four-month postoperative visit he was complaining of pain with daily activities and imaging studies showed a recurrence with increased size of the lesion (Figures 3a, 3b). The patient was subsequently taken to the operating room for a second surgical resection and planned OCA approximately eight months after his index procedure.
44 The Iowa Orthopaedic Journal
A medial parapatellar arthrotomy was performed and the knee was hyper-flexed, revealing a crown-shaped lesion that had projections both anteriorly and posteriorly and was approximately 15mm in its largest dimension. The lesion was removed with a curette, a burr, and a rongeur until on fluoroscopy the lesion was completely resected. A 15-mm, fresh, adult, press fit medial femoral condyle allograft plug was then placed, filling 95% of the lesion
Dysplasia Epiphysealis Hemimelica Treated With Osteochondral Allograft
5b
A
B 5a
6a
C
Figures 5a, 5b. Postoperative radiographs at 6-month follow-up from the patient’s OCA placement. Good graft incorporation is seen and no bony defect appreciated.
6b
Figures 6a, 6b. Radiographs at approximate 15-month follow-up demonstrate continued incorporation of the graft with an improved medial femoral condylar surface.
(Figures 4a, 4b). Standard clinical and radiographic follow-up was performed (Figures 5a, 5b; 6a, 6b). At 27 months follow-up after placement of the OCA, plain films (Figures 7a, 7b) and MRI (Figures 8a, 8b) demonstrated a cartilage surface of the medial femoral condyle that was grossly congruent without significant step-off. Additionally, the patient was pain free, had symmetric range of motion to the contralateral knee, and was participating in full activities.
DISCUSSION We present the case of a five year old boy with a two year history of right knee pain and evidence of DEH who underwent initial arthroscopic resection and subsequent revision surgery with further resection and placement of an OCA. To the best of our knowledge, this is the first reported case of a DEH lesion treated with osteochondral allograft and also the youngest reported case of osteochondral allograft placement in the literature. Volume 35 45
C. A. Anthony, B. R. Wolf
7a
7b
Figures 7a, 7b. Radiograph images at 27-month follow-up from OCA placement. Graft shows congruent joint surface of the medial femoral condyle.
The patient’s clear history of worsening pain and limp over a two year period prompted surgical intervention as it was determined in discussion with the patient’s family that continued non-operative management was no longer appropriate; however, previous authors have reported three cases of treating medial femoral condyle DEH lesions non-operatively with good outcomes (Ages 1yo, 2yo, 13yo)9, 10. Our initial approach was surgical resection. In our patient’s case, the index procedure was complicated by a portion of the lesion being located in a more anterior position on the femoral condyle. As such, the decision was made not to remove this aspect of the lesion as it was positioned in a weight-bearing portion of the knee. We acknowledge that incomplete removal of the DEH lesion in the index procedure may have contributed to the disease recurrence, though previous reports have indicated that partially resected lesions have not returned26. Fasting et al. reported treating two medial femoral condyle DEH lesions with resection through bone. In the case of a four year old female at time of resection, the patient had no complaints regarding her knee, radiographic exam showed some flattening of the joint surface of the knee and the involved limb was two cm longer than the contralateral limb at 19 year followup. In the other case (five year old male), the patient was asymptomatic at one year follow-up27. Keret et al. showed that simple excision of extra-articular lesions yielded favorable results, whereas surgical osteotomy may be required to correct angular deformities in cases of intra-articular lesions12. In their case series of nine patients, Kuo et al. reported good outcomes with surgical removal of juxta-articular lesions; however, they reported 46 The Iowa Orthopaedic Journal
fair to poor outcomes with removal of articular lesions and recommended against removal of these lesions unless they became loose bodies10. Skripitz et al reported two cases (ages two and four years old) of symptomatic medial femoral condyle DEH lesions that were treated with excision chondroplasty11. At 4.5 years follow-up, the two-year-old boy was found to have moderate restriction of range of motion in the operative knee and a leg length discrepancy of 1.5 cm lengthening in the operative leg, without pain11. At four years follow-up, the four-yearold patient was found to have a slight valgus deformity, normal range of motion, and a leg length discrepancy of one cm of lengthening of the operative side11. In the revision procedure, a complete resection of the DEH lesion recurrence was performed and a bony allograft was placed. Due to the size of the lesion removed, it was determined that placement of a graft was necessary to maintain appropriate joint congruity. As no juvenile allograft options were available, we used an adult fresh osteochondral allograft that had recently been processed by a hospital tissue vendor. Standard testing was completed and the graft was implanted less than 28 days after harvest. At 27 month follow-up after his second procedure, the patient was found to be walking and running with no pain and only occasional left knee stiffness. Plain radiographs (Figures 7a, 7b) and MRI (Figures 8a, 8b) showed near symmetric incorporation of the OCA at 27 months. There is limited data regarding knee OCA use in the pediatric and adolescent populations. Murphy et al. reported on 39 patients (43 knees) under the age of 18 (range 11-17.9 years old) who underwent knee
Dysplasia Epiphysealis Hemimelica Treated With Osteochondral Allograft
8a
8b
Figures 8a, 8b. MRI images at 27-month follow-up from OCA placement demonstrate gross congruity of cartilagenous surface.
OCA procedures (18 medial femoral condyle, 15 lateral femoral condyle, 3 patella, 2 trochlea, 1 tibial plateau, 4 multiple sites) where 12% of the knees had failure of the allograft at a median of 2.7 years (range 1.0-14.7 years)25. These five failed allografts underwent salvage OCA transplantation with 80% of the revision grafts still in place at last follow-up (follow-up range 2.3-8.8 years)25. Overall, allograft survivorship was 90% at 10 years and at final follow-up 88% of patients reported they were either “extremely satisfied” (74%) or “satisfied” (14%) with their outcome25. In their case series of 11 patients (age range 13-20 yo; 4 medial femoral condyle, 7 lateral femoral condyle, 1 trochlea, 1 patella) who underwent OCA procedures after failed initial treatment for OCD, Lyon et al. reported no graft failures and found that 82% of patients had returned to full sporting activities between 9-12 months after surgery and that all patients had returned to full daily activity levels with decreased pain24. Notably, we were unable to find any previous reports of using OCA in cases of DEH. Osteochondral autograft transplantation and autologous cartilage implantation were other potential options considered. Previous reports have described nonoperative and operative treatment for DEH lesions of the pediatric knee. We report a case of DEH of the medial femoral condyle treated with osteochondral allograft. At two year follow-up after OCA placement, the patient is asymptomatic, has returned to full activity, and has a largely normal radiographic exam. We submit OCA is a potential treatment option for patient with intra-articular DEH requiring extensive surgical excision.
REFERENCES 1. Azouz EM, Slomic AM, Marton D. The variable manifestations of dysplasia epiphysealis hemimelica. PediatrRadiol 1985; 15: 44-9. 2. Rosero VM, Kiss S, Terebessy T, et al. Dysplasia epiphysealis hemimelica (Trevor’s disease): 7 of our own cases and a review of the literature. Acta Orthop 2007;78:856e61. 3. Tyler PA, Rajeswaran G, Saifuddin A. Imaging of dysplasia epiphysealis hemimelica (Trevor’s disease). Clin Radiol. 2013 Apr;68(4):415-21. 4. Mouchet A, Belot J. Tarso megalie. J Radiol Electrol 1926;10:289e93. 5. Trevor D. Tarso-epiphysial aclasis; a congenital error of epiphysial development. J Bone Joint Surg Br. 1950 May;32-B(2):204-13. 6. Acquaviva A, Municchi G, Marconcini S, Mazzarella F, Occhini R, Toti P, Mazzei M A, Volterrani L. Dysplasia epiphysealis hemimelica in a young girl: role of MRI in the diagnosis and follow-up. Joint Bone Spine 2005; 72:183-6. 7. Kircher J, Westhoff B, Bittersohl B, Ziskoven C, Krauspe R. A rare case of Trevor’s disease (dysplasia epiphysealis hemimelica) in the elbow. J Shoulder Elbow Surg. 2013 Feb;22(2):e12-5. 8. Bovee JV, Hameetman L, Kroon HM, et al. EXTrelated pathways are not involved in the pathogenesis of dysplasia epiphysealis hemimelica and metachondromatosis. J Pathol 2006;209:411e9. 9. Perl M, Brenner RE, Lippacher S, et al. Dysplasia epiphysealis hemimelica: a case report with novel pathophysiologic aspects. Clin Orthop Relat Res 2009;467:2472e8. Volume 35 47
C. A. Anthony, B. R. Wolf 10. Kuo RS, Bellemore MC, Monsell FP, Frawley K, Kozlowski K. Dysplasia epiphysealis hemimelica: clinical features and management. J Pediatr Orthop. 1998 Jul-Aug;18(4):543-8. 11. Skripitz R, Lüssenhop S, Meiss AL. Wedge excision chondroplasty of the knee in dysplasiaepiphysealis hemimelica—report of 2 cases. Acta Orthop Scand. 2003 Apr;74(2):225-9. 12. Keret D, Spatz DK, Caro PA, Mason DE. Dysplasia epiphysealis hemimelica: diagnosis and treatment. J Pediatr Orthop. 1992 May-Jun;12(3):365-72. 13. Lorani A, Huff D, Dormans JP. Multiple masses in a 2-year 5-month old girl. Clin Orthop Relat Res. 2000;375:320–323, 338–340. 14. Emmerson BC, Görtz S, Jamali AA, Chung C, Amiel D, Bugbee WD. Fresh osteochondral allografting in the treatment of osteochondritis dissecans of the femoral condyle. Am J Sports Med 2007;35(6):907-914. 15. Ghazavi MT, Pritzker KP, Davis AM, Gross AE. Fresh osteochondral allografts for post-traumatic osteochondral defects of the knee. J Bone Joint Surg Br 1997; 79(6):1008-1013. 16. Görtz S, De Young AJ, Bugbee WD. Fresh osteochondral allografting for steroid-associated osteonecrosis of the femoral condyles. Clin Orthop Relat Res 2010;468(5):1269-1278. 17. Gross AE, Shasha N, Aubin P. Longterm followup of the use of fresh osteochondral allografts for posttraumatic knee defects. Clin Orthop Relat Res 2005;435:79-87. 18. Levy YD, Görtz S, Pulido PA, McCauley JC, Bugbee WD. Do fresh osteochondral allografts successfully treat femoral condyle lesions? Clin Orthop Relat Res 2013;471(1):231-237. 19. Sherman SL, Garrity J, Bauer K, Cook J, Stannard J, Bugbee W. Fresh osteochondral allograft transplantation for the knee: current concepts. J Am Acad Orthop Surg. 2014 Feb;22(2):121-33.
48 The Iowa Orthopaedic Journal
20. Amiel D, Har wood FL, Hoover JA, Meyers M. A histological and biochemical assessment of the cartilage matrix obtained from in vitro storage of osteochondral allografts. Connect Tissue Res 1989;23(1):89-99. 21. Garrett JC. Fresh osteochondral allografts for treatment of articular defects in osteochondritis dissecans of the lateral femoral condyle in adults. Clin Orthop Relat Res 1994;303:33-37. 22. Jamali AA, Emmerson BC, Chung C, Conver y FR, Bugbee WD. Fresh osteochondral allografts: Results in the patellofemoral joint. Clin Orthop Relat Res 2005;437:176-185. 23. Rue JP, Yanke AB, Busam ML, McNickle AG, Cole BJ. Prospective evaluation of concurrent meniscus transplantation and articular cartilage repair: Minimum 2-year follow-up. Am J Sports Med 2008;36(9):1770-1778. 24. Lyon R, Nissen C, Liu XC, Curtin B. Can fresh osteochondral allografts restore function in juveniles with osteochondritis dissecans of the knee? Clin Orthop Relat Res. 2013 Apr;471(4):1166-73. 25. Murphy RT, Pennock AT, Bugbee WD. Osteochondral allograft transplantation of the knee in the pediatric and adolescent population. Am J Sports Med. 2014 Mar;42(3):635-40. 26. Bahk WJ, Lee HY, Kang YK, Park JM, Chun KA, Chung YG. Dysplasia epiphysealis hemimelica: radiographic and magnetic resonance imaging features and clinical outcome of complete and incomplete resection. Skeletal Radiol. 2010 Jan;39(1):85-90. 27. Fasting OJ, Bjerkreim I. Dysplasia epiphysealis hemimelica. Acta Orthop Scand. 1976 Apr;47(2):21725.
ABSTRACT Background: Dysplasia epiphysealis hemimelica (DEH), or Trevor’s disease, is a developmental disorder of the pediatric skeleton characterized by asymmetric osteochondral overgrowth. Methods: We present the case of a five year old boy with a two year histor y of right knee pain and evidence of DEH on imaging who underwent initial arthroscopic resection of his lesion with subsequent recurrence. The patient then underwent osteochondral allograft revision surger y and was asymptomatic at two year follow-up with a congruent joint surface. Results: To our knowledge, this is the first reported case of a DEH lesion treated with osteochondral allograft and also the youngest reported case of osteochondral allograft placement in the literature. Conclusions: Osteochondral allograft may be a viable option in DEH and other deformities of the pediatric knee. Level of Evidence: Level V INTRODUCTION Dysplasia epiphysealis hemimelica (DEH), or Trevor’s disease, is a developmental disorder of the skeleton most often characterized by asymmetric osteochondral overgrowth1-3. DEH was initially reported in 1926 and was further described by Trevor in 19504,5. The disease is rare with a reported incidence of one per million and a male predominance with a male: female ratio ranging from 2:1 to 3:12,6. The distal femur, distal tibia and fibula, The Department of Orthopaedic Surgery and Rehabilitation, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, 01008 JPP, Iowa City, IA 52242, USA Email: [email protected] Phone: 563-343-1951 Each author certifies that his or her institution approved or waived approval for the human protocol for this investigation and that all investigations were conducted in conformity with ethical principles of research. Each author certifies that he or she, or a member of his or her immediate family, has no funding or commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the current article.
42 The Iowa Orthopaedic Journal
talus and calcaneus are most often affected with lesions involving the carpal bones, bones of the midfoot, scapula, acetabulum and elbow also reported2,7. DEH can affect either the medial or lateral epiphysis, though the medial epiphysis is most commonly involved1-3. Clinical findings typically include deformity, pain, limb-length discrepancy, muscle wasting, swelling, and limitations in joint range of motion2,3. Histologically the lesion is similar to an osteochondroma, however, DEH lacks the EXT1 and EXT2 gene mutations seen in osteochondroma8,9. DEH is also classically an epiphyseal lesion whereas osteochondroma is more commonly metaphyseal in location3. On plain radiographs the typical appearance of DEH is asymmetric epiphyseal cartilaginous overgrowth, containing more than one ossification center, with varying patterns of epiphyseal chondral calcification3. Computed tomography can be used for the detection of small foci of early calcification within the cartilaginous mass of a DEH lesion and can identify cortical and medullary continuity between the lesion and the neighboring bone3. Additionally, MRI can be useful in characterizing the size and dimensions of the lesion before it begins to ossifiy3. Management options of DEH include observation, surgical excision, and corrective osteotomy3, 10, 11. Surgical removal is indicated if the lesion is causing deformity, pain, compromising function, or resulting in an intraarticular loose body5, 12, 13. Osteochondral allograft (OCA) is an accepted method of treatment for restoring large chondral and osteochondral defects on the femoral condyles. Previous work has found survival rates of OCA in cases of idiopathic, focal chondral or osteochondral lesions of the femoral condyles ranged from 85%-100% at 5 years, 71%-89% at 10 years, 74%-76% at up to 15 years, and 66% at up to 20 years14-19. In cases of osteochondritis dissecans (OCD) and steroid associated osteonecrosis, OCA survival rates of 79% to 94% have been seen at less than five years follow-up19-23. There is limited data regarding OCA use in the pediatric knee. Published results have shown that 82% of patients returned to full sporting activities by twelve months24 and a graft survivorship of 90% at ten years25. We present the case of a five year old boy who underwent arthroscopic resection for a symptomatic left medial femoral condyle DEH lesion and subsequent
Dysplasia Epiphysealis Hemimelica Treated With Osteochondral Allograft
1a
1b
Figures 1a, 1b. Initial preoperative radiographs of the left knee which demonstrate an irregular radiodensity of the condylar surface along the posterior aspect of the medial femoral condyle.
2a
2b
Figures 2a, 2b. Radiographs of the articular surface of the left knee immediately after initial arthroscopic debridement of the DEH lesion. Of note, there is approximately 10% of the anterior portion of the lesion that was not removed as it was in the weightbearing zone of the medial femoral condyle.
repeat resection and placement of OCA due to lesion recurrence. The patient’s course with two year follow-up including history, exam, radiology findings, and treatment are discussed. CASE REPORT A five year old boy was referred to orthopaedics clinic by his local pediatrician with a two year history of worsening left knee pain and limp. Radiographs of the
knee were consistent with DEH of the left medial femoral condyle (Figures 1a, 1b). During initial arthroscopic resection, there was noted to be a prominence of the articular surface on the posterior aspect of the medial femoral condyle. The cartilage overlying the lesion was taken down with a shaver and the bony prominence within the epiphysis was identified and resected. Approximately 80-90% of the lesion was removed while the anterior-most portion of the lesion was left undisturbed Volume 35 43
C. A. Anthony, B. R. Wolf
3a
3b
Figures 3a, 3b. Radiographs demonstrating the recurrence of an irregular, crown shaped lesion along the posterior aspect of the medial femoral condyle at approximately 4 months after the index procedure.
A
4a
4b
Figures 4a, 4b. Immediate postoperative radiographs after placement of the adult osteochondral allograft.
as it was distinctly in the weightbearing zone of the knee (Figures 2a, 2b). At the patient’s four-month postoperative visit he was complaining of pain with daily activities and imaging studies showed a recurrence with increased size of the lesion (Figures 3a, 3b). The patient was subsequently taken to the operating room for a second surgical resection and planned OCA approximately eight months after his index procedure.
44 The Iowa Orthopaedic Journal
A medial parapatellar arthrotomy was performed and the knee was hyper-flexed, revealing a crown-shaped lesion that had projections both anteriorly and posteriorly and was approximately 15mm in its largest dimension. The lesion was removed with a curette, a burr, and a rongeur until on fluoroscopy the lesion was completely resected. A 15-mm, fresh, adult, press fit medial femoral condyle allograft plug was then placed, filling 95% of the lesion
Dysplasia Epiphysealis Hemimelica Treated With Osteochondral Allograft
5b
A
B 5a
6a
C
Figures 5a, 5b. Postoperative radiographs at 6-month follow-up from the patient’s OCA placement. Good graft incorporation is seen and no bony defect appreciated.
6b
Figures 6a, 6b. Radiographs at approximate 15-month follow-up demonstrate continued incorporation of the graft with an improved medial femoral condylar surface.
(Figures 4a, 4b). Standard clinical and radiographic follow-up was performed (Figures 5a, 5b; 6a, 6b). At 27 months follow-up after placement of the OCA, plain films (Figures 7a, 7b) and MRI (Figures 8a, 8b) demonstrated a cartilage surface of the medial femoral condyle that was grossly congruent without significant step-off. Additionally, the patient was pain free, had symmetric range of motion to the contralateral knee, and was participating in full activities.
DISCUSSION We present the case of a five year old boy with a two year history of right knee pain and evidence of DEH who underwent initial arthroscopic resection and subsequent revision surgery with further resection and placement of an OCA. To the best of our knowledge, this is the first reported case of a DEH lesion treated with osteochondral allograft and also the youngest reported case of osteochondral allograft placement in the literature. Volume 35 45
C. A. Anthony, B. R. Wolf
7a
7b
Figures 7a, 7b. Radiograph images at 27-month follow-up from OCA placement. Graft shows congruent joint surface of the medial femoral condyle.
The patient’s clear history of worsening pain and limp over a two year period prompted surgical intervention as it was determined in discussion with the patient’s family that continued non-operative management was no longer appropriate; however, previous authors have reported three cases of treating medial femoral condyle DEH lesions non-operatively with good outcomes (Ages 1yo, 2yo, 13yo)9, 10. Our initial approach was surgical resection. In our patient’s case, the index procedure was complicated by a portion of the lesion being located in a more anterior position on the femoral condyle. As such, the decision was made not to remove this aspect of the lesion as it was positioned in a weight-bearing portion of the knee. We acknowledge that incomplete removal of the DEH lesion in the index procedure may have contributed to the disease recurrence, though previous reports have indicated that partially resected lesions have not returned26. Fasting et al. reported treating two medial femoral condyle DEH lesions with resection through bone. In the case of a four year old female at time of resection, the patient had no complaints regarding her knee, radiographic exam showed some flattening of the joint surface of the knee and the involved limb was two cm longer than the contralateral limb at 19 year followup. In the other case (five year old male), the patient was asymptomatic at one year follow-up27. Keret et al. showed that simple excision of extra-articular lesions yielded favorable results, whereas surgical osteotomy may be required to correct angular deformities in cases of intra-articular lesions12. In their case series of nine patients, Kuo et al. reported good outcomes with surgical removal of juxta-articular lesions; however, they reported 46 The Iowa Orthopaedic Journal
fair to poor outcomes with removal of articular lesions and recommended against removal of these lesions unless they became loose bodies10. Skripitz et al reported two cases (ages two and four years old) of symptomatic medial femoral condyle DEH lesions that were treated with excision chondroplasty11. At 4.5 years follow-up, the two-year-old boy was found to have moderate restriction of range of motion in the operative knee and a leg length discrepancy of 1.5 cm lengthening in the operative leg, without pain11. At four years follow-up, the four-yearold patient was found to have a slight valgus deformity, normal range of motion, and a leg length discrepancy of one cm of lengthening of the operative side11. In the revision procedure, a complete resection of the DEH lesion recurrence was performed and a bony allograft was placed. Due to the size of the lesion removed, it was determined that placement of a graft was necessary to maintain appropriate joint congruity. As no juvenile allograft options were available, we used an adult fresh osteochondral allograft that had recently been processed by a hospital tissue vendor. Standard testing was completed and the graft was implanted less than 28 days after harvest. At 27 month follow-up after his second procedure, the patient was found to be walking and running with no pain and only occasional left knee stiffness. Plain radiographs (Figures 7a, 7b) and MRI (Figures 8a, 8b) showed near symmetric incorporation of the OCA at 27 months. There is limited data regarding knee OCA use in the pediatric and adolescent populations. Murphy et al. reported on 39 patients (43 knees) under the age of 18 (range 11-17.9 years old) who underwent knee
Dysplasia Epiphysealis Hemimelica Treated With Osteochondral Allograft
8a
8b
Figures 8a, 8b. MRI images at 27-month follow-up from OCA placement demonstrate gross congruity of cartilagenous surface.
OCA procedures (18 medial femoral condyle, 15 lateral femoral condyle, 3 patella, 2 trochlea, 1 tibial plateau, 4 multiple sites) where 12% of the knees had failure of the allograft at a median of 2.7 years (range 1.0-14.7 years)25. These five failed allografts underwent salvage OCA transplantation with 80% of the revision grafts still in place at last follow-up (follow-up range 2.3-8.8 years)25. Overall, allograft survivorship was 90% at 10 years and at final follow-up 88% of patients reported they were either “extremely satisfied” (74%) or “satisfied” (14%) with their outcome25. In their case series of 11 patients (age range 13-20 yo; 4 medial femoral condyle, 7 lateral femoral condyle, 1 trochlea, 1 patella) who underwent OCA procedures after failed initial treatment for OCD, Lyon et al. reported no graft failures and found that 82% of patients had returned to full sporting activities between 9-12 months after surgery and that all patients had returned to full daily activity levels with decreased pain24. Notably, we were unable to find any previous reports of using OCA in cases of DEH. Osteochondral autograft transplantation and autologous cartilage implantation were other potential options considered. Previous reports have described nonoperative and operative treatment for DEH lesions of the pediatric knee. We report a case of DEH of the medial femoral condyle treated with osteochondral allograft. At two year follow-up after OCA placement, the patient is asymptomatic, has returned to full activity, and has a largely normal radiographic exam. We submit OCA is a potential treatment option for patient with intra-articular DEH requiring extensive surgical excision.
REFERENCES 1. Azouz EM, Slomic AM, Marton D. The variable manifestations of dysplasia epiphysealis hemimelica. PediatrRadiol 1985; 15: 44-9. 2. Rosero VM, Kiss S, Terebessy T, et al. Dysplasia epiphysealis hemimelica (Trevor’s disease): 7 of our own cases and a review of the literature. Acta Orthop 2007;78:856e61. 3. Tyler PA, Rajeswaran G, Saifuddin A. Imaging of dysplasia epiphysealis hemimelica (Trevor’s disease). Clin Radiol. 2013 Apr;68(4):415-21. 4. Mouchet A, Belot J. Tarso megalie. J Radiol Electrol 1926;10:289e93. 5. Trevor D. Tarso-epiphysial aclasis; a congenital error of epiphysial development. J Bone Joint Surg Br. 1950 May;32-B(2):204-13. 6. Acquaviva A, Municchi G, Marconcini S, Mazzarella F, Occhini R, Toti P, Mazzei M A, Volterrani L. Dysplasia epiphysealis hemimelica in a young girl: role of MRI in the diagnosis and follow-up. Joint Bone Spine 2005; 72:183-6. 7. Kircher J, Westhoff B, Bittersohl B, Ziskoven C, Krauspe R. A rare case of Trevor’s disease (dysplasia epiphysealis hemimelica) in the elbow. J Shoulder Elbow Surg. 2013 Feb;22(2):e12-5. 8. Bovee JV, Hameetman L, Kroon HM, et al. EXTrelated pathways are not involved in the pathogenesis of dysplasia epiphysealis hemimelica and metachondromatosis. J Pathol 2006;209:411e9. 9. Perl M, Brenner RE, Lippacher S, et al. Dysplasia epiphysealis hemimelica: a case report with novel pathophysiologic aspects. Clin Orthop Relat Res 2009;467:2472e8. Volume 35 47
C. A. Anthony, B. R. Wolf 10. Kuo RS, Bellemore MC, Monsell FP, Frawley K, Kozlowski K. Dysplasia epiphysealis hemimelica: clinical features and management. J Pediatr Orthop. 1998 Jul-Aug;18(4):543-8. 11. Skripitz R, Lüssenhop S, Meiss AL. Wedge excision chondroplasty of the knee in dysplasiaepiphysealis hemimelica—report of 2 cases. Acta Orthop Scand. 2003 Apr;74(2):225-9. 12. Keret D, Spatz DK, Caro PA, Mason DE. Dysplasia epiphysealis hemimelica: diagnosis and treatment. J Pediatr Orthop. 1992 May-Jun;12(3):365-72. 13. Lorani A, Huff D, Dormans JP. Multiple masses in a 2-year 5-month old girl. Clin Orthop Relat Res. 2000;375:320–323, 338–340. 14. Emmerson BC, Görtz S, Jamali AA, Chung C, Amiel D, Bugbee WD. Fresh osteochondral allografting in the treatment of osteochondritis dissecans of the femoral condyle. Am J Sports Med 2007;35(6):907-914. 15. Ghazavi MT, Pritzker KP, Davis AM, Gross AE. Fresh osteochondral allografts for post-traumatic osteochondral defects of the knee. J Bone Joint Surg Br 1997; 79(6):1008-1013. 16. Görtz S, De Young AJ, Bugbee WD. Fresh osteochondral allografting for steroid-associated osteonecrosis of the femoral condyles. Clin Orthop Relat Res 2010;468(5):1269-1278. 17. Gross AE, Shasha N, Aubin P. Longterm followup of the use of fresh osteochondral allografts for posttraumatic knee defects. Clin Orthop Relat Res 2005;435:79-87. 18. Levy YD, Görtz S, Pulido PA, McCauley JC, Bugbee WD. Do fresh osteochondral allografts successfully treat femoral condyle lesions? Clin Orthop Relat Res 2013;471(1):231-237. 19. Sherman SL, Garrity J, Bauer K, Cook J, Stannard J, Bugbee W. Fresh osteochondral allograft transplantation for the knee: current concepts. J Am Acad Orthop Surg. 2014 Feb;22(2):121-33.
48 The Iowa Orthopaedic Journal
20. Amiel D, Har wood FL, Hoover JA, Meyers M. A histological and biochemical assessment of the cartilage matrix obtained from in vitro storage of osteochondral allografts. Connect Tissue Res 1989;23(1):89-99. 21. Garrett JC. Fresh osteochondral allografts for treatment of articular defects in osteochondritis dissecans of the lateral femoral condyle in adults. Clin Orthop Relat Res 1994;303:33-37. 22. Jamali AA, Emmerson BC, Chung C, Conver y FR, Bugbee WD. Fresh osteochondral allografts: Results in the patellofemoral joint. Clin Orthop Relat Res 2005;437:176-185. 23. Rue JP, Yanke AB, Busam ML, McNickle AG, Cole BJ. Prospective evaluation of concurrent meniscus transplantation and articular cartilage repair: Minimum 2-year follow-up. Am J Sports Med 2008;36(9):1770-1778. 24. Lyon R, Nissen C, Liu XC, Curtin B. Can fresh osteochondral allografts restore function in juveniles with osteochondritis dissecans of the knee? Clin Orthop Relat Res. 2013 Apr;471(4):1166-73. 25. Murphy RT, Pennock AT, Bugbee WD. Osteochondral allograft transplantation of the knee in the pediatric and adolescent population. Am J Sports Med. 2014 Mar;42(3):635-40. 26. Bahk WJ, Lee HY, Kang YK, Park JM, Chun KA, Chung YG. Dysplasia epiphysealis hemimelica: radiographic and magnetic resonance imaging features and clinical outcome of complete and incomplete resection. Skeletal Radiol. 2010 Jan;39(1):85-90. 27. Fasting OJ, Bjerkreim I. Dysplasia epiphysealis hemimelica. Acta Orthop Scand. 1976 Apr;47(2):21725.
